Supplementary Material for: Surgical treatment of Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep associated with polymicrogyria: A case report

Introduction:Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep (EE-SWAS) is a rare childhood-onset epileptic encephalopathy, defined by a typical electrographic pattern with mostly continuous slow spike-wave complexes during non-REM sleep. It may be cryptogenic or secondary to genetic or structural etiology. It might be related to progressive and irreversible cognitive deterioration; under these circumstances, there is an urgency for fast adequate treatment. Polymicrogyria is a common brain malformation and epilepsy can occur in up to 90% of the patients. Extensive polymicrogyria can be a cause of EE-SWAS and treatment refractoriness. In these patients, surgical treatment might be considered. Case Presentation: We report on an eight-year-old child who presented with electrographic findings compatible with EE-SWAS and hemiparesis associated with right hemispheric polymicrogyria who successfully underwent hemispherectomy as primary treatment. Surgery is rarely considered for these patients. Conclusion: This report highlights the importance of considering surgery as an early treatment modality in selected cases引言：睡眠期棘慢波激活性癫痫性脑病（Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep, EE-SWAS）是一种罕见的儿童起病型癫痫性脑病，其诊断标准为非快速眼动（non-REM）睡眠期存在以连续性慢棘慢波复合波为主的典型脑电图表现。该病病因可分为隐源性，或继发于遗传或结构性病因。该病可伴发进行性且不可逆的认知功能衰退，此类情况下需尽快实施恰当的治疗。多小脑回畸形是一种常见的脑发育畸形，约90%的患者可伴发癫痫。广泛性多小脑回畸形可诱发EE-SWAS并导致治疗抵抗，此类患者可考虑接受外科手术治疗。 病例报告：本文报告1例8岁患儿，其脑电图结果符合EE-SWAS诊断，且存在右侧半球多小脑回畸形相关的偏瘫，该患儿以大脑半球切除术作为初始治疗并获得成功。此类患者通常极少考虑手术治疗。 结论：本案例报告强调，在符合遴选指征的病例中，将手术作为早期治疗手段具有重要临床价值。